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Kaposi's sarcoma: Etiology and pathogenesis, inducing factors, causal associations, and treatments: Facts and controversies

机译:卡波济肉瘤:病因和发病机制,诱发因素,因果关系和治疗:事实和争议

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摘要

Kaposi's sarcoma (KS), an angioproliferative disorder, has a viral etiology and a multifactorial pathogenesis hinged on an immune dysfunction. The disease is multifocal, with a course ranging from indolent, with only skin manifestations to fulminant, with extensive visceral involvement. In the current view, all forms of KS have a common etiology in human herpesvirus (HHV)-8 infection, and the differences among them are due to the involvement of various cofactors. In fact, HHV-8 infection can be considered a necessary but not sufficient condition for the development of KS, because further factors (genetic, immunologic, and environmental) are required. The role of cofactors can be attributed to their ability to interact with HHV-8, to affect the immune system, or to act as vasoactive agents. In this contribution, a survey of the current state of knowledge on many and various factors involved in KS pathogenesis is carried out, in particular by highlighting the facts and controversies about the role of some drugs (quinine analogues and angiotensin-converting enzyme inhibitors) in the onset of the disease. Based on these assessments, it is possible to hypothesize that the role of cofactors in KS pathogenesis can move toward an effect either favoring or inhibiting the onset of the disease, depending on the presence of other agents modulating the pathogenesis itself, such as genetic predisposition, environmental factors, drug intake, or lymph flow disorders. It is possible that the same agents may act as either stimulating or inhibiting cofactors according to the patient's genetic background and variable interactions. Treatment guidelines for each form of KS are outlined, because a unique standard therapy for all of them cannot be considered due to KS heterogeneity. In most cases, therapeutic options, both local and systemic, should be tailored to the patient's peculiar clinical conditions.
机译:卡波济肉瘤(KS)是一种血管增生性疾病,其病毒病因和多因素发病机制取决于免疫功能障碍。该病是多灶性的,病程从轻度,仅皮肤表现到暴发,内脏受累广泛。当前,所有形式的KS在人类疱疹病毒(HHV)-8感染中都有共同的病因,并且它们之间的差异是由于各种辅助因子的参与所致。实际上,由于需要进一步的因素(遗传,免疫和环境),因此可以将HHV-8感染视为KS发生的必要但不充分的条件。辅因子的作用可以归因于它们与HHV-8相互作用,影响免疫系统或充当血管活性剂的能力。在这项贡献中,对KS发病机理涉及的许多因素的知识现状进行了调查,特别是着重强调了某些药物(奎宁类似物和血管紧张素转化酶抑制剂)的作用的事实和争议。这种疾病的发作。根据这些评估,可以假设辅因子在KS发病机理中的作用可能趋向于促进或抑制疾病发作的效果,这取决于是否存在其他调节发病机理的因素,例如遗传易感性,环境因素,药物摄入或淋巴流动异常。根据患者的遗传背景和可变的相互作用,相同的药物可能会起到刺激或抑制辅助因子的作用。概述了每种形式的KS的治疗指南,因为由于KS异质性,不能考虑针对所有这些形式的独特标准疗法。在大多数情况下,应根据患者的特殊临床情况调整局部和全身治疗选择。

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